Pheochromocytoma is a catecholamine-secreting neuroendocrine tumor arising from chromaffin cells of the adrenal medulla. Extra-adrenal tumors = paragangliomas (arise from sympathetic ganglia -organ of Zuckerkandl most common). Classic presentation: paroxysmal triad of headache + sweating + palpitations with hypertension. Accounts for < 1% of hypertension but must be considered in: resistant HTN, hypertensive crisis with paroxysms, adrenal incidentaloma, familial syndromes (MEN2A/2B, VHL, NF1, SDH mutations). "Rule of 10s": 10% bilateral, 10% extra-adrenal, 10% malignant, 10% pediatric, ~40% familial (higher than classically taught). The critical teaching point: Alpha-blockade MUST precede beta-blockade -unopposed alpha stimulation during beta-blockade → hypertensive crisis.
| Drug | Dose | Route | Notes |
|---|---|---|---|
| Phenoxybenzamine | 10 mg BID → titrate to 20-30 mg BID | PO | Non-competitive alpha-blocker. Start 10-14 days pre-op. Irreversible -long duration. Orthostatic hypotension, nasal congestion, reflex tachycardia. |
| Doxazosin | 2-8 mg daily | PO | Competitive alpha-1 blocker. Shorter acting than phenoxybenzamine. Less tachycardia. Some centers prefer. |
| Propranolol | 20-40 mg TID | PO | ONLY after alpha-blockade established. Controls reflex tachycardia. Start 2-3 days before surgery. |
| Metyrosine | 250 mg QID (max 4g/day) | PO | Tyrosine hydroxylase inhibitor -blocks catecholamine synthesis. For refractory hypertension or inoperable tumors. Sedation, EPS side effects. |
| Phentolamine | 2-5 mg IV q5min PRN | IV | Hypertensive crisis. Competitive alpha-blocker. Fast onset. Have ready in OR during tumor manipulation. |
| Nicardipine | 5-15 mg/hr IV | IV drip | Alternative for intraoperative/crisis BP control. Smooth, titratable. |
Patient: 38F with episodic headaches, diaphoresis, and palpitations × 4 months. Episodes last 20-30 min, occur 3-4×/week. BP 230/120 during episode, 140/88 at baseline. HR 128 during episode. Resistant to 3 antihypertensives.
Key findings: Classic paroxysmal triad (headache + diaphoresis + palpitations). Plasma free metanephrines: metanephrine 420 pg/mL (elevated), normetanephrine 2100 pg/mL (markedly elevated). CT: 3.5 cm right adrenal mass, 35 HU.
Management:
Teaching point: Alpha before beta, the cardinal rule of pheo management. Beta-blockers without alpha-blockade remove beta-2 vasodilation, leaving unopposed alpha vasoconstriction, causing severe hypertensive crisis.
Patient: 52M undergoing cholecystectomy. During insufflation, sudden BP 280/160, HR 160, SVT on telemetry. No known pheo history. Incidental 2.8 cm adrenal mass seen on pre-op CT but not investigated.
Key findings: Intraoperative catecholamine crisis, triggered by abdominal insufflation compressing an undiagnosed pheochromocytoma. Surgical manipulation is the classic precipitant of pheo crisis.
Management:
Teaching point: Adrenal incidentalomas > 1 cm should always have plasma metanephrines checked before any surgery. An undiagnosed pheo in the OR has a mortality rate of up to 80% if unrecognized.
Patient: 24M with HTN and headaches. Father had bilateral pheos at age 30. Plasma normetanephrine 980 pg/mL. MRI: 2.2 cm left extra-adrenal retroperitoneal mass (paraganglioma). Right adrenal normal.
Key findings: Young age + family history + extra-adrenal location = high suspicion for hereditary syndrome. ~40% of pheos/paragangliomas have germline mutations (SDHx, VHL, MEN2, NF1).
Management:
Teaching point: All pheos/paragangliomas should get genetic testing, 40% are hereditary. SDHx mutations carry the highest malignancy risk (up to 40% for SDHB). Extra-adrenal location and young age are red flags for hereditary syndromes.
Mr. Park is a 42-year-old man presenting with episodic headaches, diaphoresis, and palpitations × 6 months. Episodes last 15-30 min, occur 2-3 times per week, associated with severe hypertension (240/130 during episodes, 155/95 baseline). Home medications: amlodipine 10 mg, lisinopril 40 mg, HCTZ 25 mg (resistant HTN). Labs: plasma normetanephrine 1850 pg/mL (> 4× ULN). CT abdomen: 4.2 cm right adrenal mass, heterogeneous enhancement, 38 HU on non-contrast.